1. Vasculitides Premchand Anne, MD, MPH PGY II (aka the class that rocks)
Internal Medicine/Pediatrics

2. Objectives basic principles of vasculitis cases/discussion/didactic
questions

3. Vasculitis Inflammation of blood vessels and its consequences
Classification
Pathology of vasculitis
Role of Immune complexes, complement, in vasculitis
Presentation: purpura vs. nodule

4. Doctor, Doctor, my son has a rash for three days.
8 year old male who is very active in sports, presents with complaints of rash and swelling of the feet and ankles. Pt’s mother noticed a progressive bumpy red rash and the swelling over the past three days. Rash is also present on buttocks bilaterally and onset similar to that of the feet. Low grade fevers for a week, no nausea or vomiting but reported frothy urine and intermittent abdominal pain over the past few hours. History of a URI in the family, including the child, 2-3 weeks ago. No family history. No PMHx.
QUESTIONS?
DIFFERENTIAL DIAGNOSES?
WORK UP?

6. Henoch Schonlein Purpura
Self limited vasculitis of the small vessels
Occurrence 9/ with male predominance
2-8 years of age, most common
IgA and C3a mediated disease and can occur with other
Renal involvement leads to nephrotic syndrome
Clinical diagnosis
Arthritis in knees and ankles
Angioedema in dependent areas with palpable lesions
Intermittent abdominal pain with possibility of intussusception (3%) with currant jelly stools.

7. Henoch Schonlein Purpura cont...
Differential diagnosis
PAN with neuro and cardiology involvement
Meningococcemia for the rash
Kawasaki disease-rash limited to LE, high fever
JRA-salmon pink rash that is fleeting
Flea and spider bites
Labs: Def. Dx: bx of lesion shows leukoclastic angiitis, Bx of renal shows IgA nephropathy and segmental GN with crescents, elevated ESR, and elevated serum IgM and IgG
Therapy: symptomatic; high dose steroids if significant GI, Neuro, and renal involvement and baby aspirin if hypercoaguble

8. Doctor, Doctor, my child has a high fever and a red tongue
3 year old Asian girl is brought in for persistent fevers of oF for more than two weeks. Patient was taken to primary pediatrician and was given antibiotics for a sorethroat 10days ago but no improvement in fevers, neck masses or the redness of the tongue and oral cavity. Fevers are improving but the girl has conjunctival injection and has periungual and perianal desquamation and incredibly irritable and poor appetite. No N/V/D/myalgias but reporting joint pains.
QUESTIONS?
DIFFERENTIAL DIAGNOSES?
WORK UP?

10. Kawasaki Disease Aka MUCOCUTANEOUS LYMPH NODE SYNDROME
febrile vasculitis of childhood
cause is unknown
unlikely in <4 months due to maternal Ab
80% are under 5 years of age
Path: severe inflammation of medium sized vessels, with strong affinity for the coronary arteries, leading to destruction of the intimal layer (all 3 layers involved in severe cases)
Cardiac disease: MOST COMMON, decreased ventricular function 2o myocarditis, pericarditis, and coronary aneurysms, thrombosis, stenosis, and sudden death.

11. Kawasaki disease cont... Clinical manifestations:
Acute phase: 1-2 weeks with fevers upto 104F and unresponsive to Antibiotics
Subacute phase: irritability, anorexia, conjuctival infection, desquamation of periungual and perineal regions and arthritis
Convalescent stage: 6-8 weeks post onset of disease
bilateral bulbar conjunctival injection without exudate
strawberry tongue and oral erythema
erythema and edema of arms and legs
CERVICAL LYMPHADENOPATHY > 1.5 cm
Rash
Differential Diagnoses:
Scarlet fever, TSSS, Measles, SJS, JRA, and RMSF

12. Kawasaki disease cont...
Labs: 2D-Echo absolutely pertinent due to extensive coronary involvement; Ig profile due to elevations in subacute phase, coag studies. Follow up 2D absolutely recommended
Therapy:
IVIG: decreases CAD by 75% (2g/kg over 10 hours)
ASA for hypercoagulable state due to thrombocytosis

13. Doctor, Doctor, I have painful marks on my legs
25 y/o asian indian female presents with complaints of nightsweats, anorexia, weight loss, fatigue, and myalgias of few weeks duration. She reports coming to the US 3 months ago. In addition to the above symptoms, pt noticed severely painful nodular lesions in the anterior aspects of her legs. Pain is unremitting. Physical exam reveals a thin women in distress secondary pain, (+) carotid bruit on the right, widened and laterally displaced PMI, and Left chest pain relieved with leaning forward. AVSS. (+) splenomegaly. LE exam with nodular lesions resembling erythema nodosum and tender to touch.
QUESTIONS?
DIFFERENTIAL DIAGNOSES?
WORKUP?

15. TAKAYASU ARTERITIS
Vasculitis affecting mostly large caliber vessels, mainly aortic arch branches
predominant in Asian and Indian subcontinent peoples, women >>men predominance.
1/3 before the age of 20
Path: preference for aorta and major branches with significant renal disease (GN). Association with TB exposure and HLA-B
Differential Diagnoses:
Cogan syndrome
Behcet’s disease
Reiter syndrome
Sarcoidosis

16. TAKAYASU ARTERITIS CONT...
Clinical manifestations:
pre-pulseless:
nightsweats
anorexia
weight loss
fatigue
myalgia
pulseless:
splenomegaly
erythema nodosum, syncope, amaurosis fugax
dilated CM, myocarditis, and pericarditis
(+) vascular bruit.
Labs: ESR>60, microcytic hypochromic anemia
Confirmation of Dx: angiography for occlusive and aneurysmal disease

17. TAKAYASU ARTERITIS CONT...
Therapy
surgical excision of stenotic lesions and management of dilated aorta if indolent disease
Immunosuppressives:
prednisone (1mg/kg/day) in early stage,
cyclophosphamide and methotrexate if refractory to steroids

18. Doctor, Doctor, my child needs your help
10 year old child presented with complaints of persistent fevers with diagnosis of FUO, diarrhea, skin lesions, numbness and tingling with muscle weakness for the past few days. Physical exam with temp of 101.1, erythematous and necrotic skin lesions on the leg and lateral aspect of the ankle, similar to stasis ulcers, and Left foot drop
QUESTIONS?
DIFFERENTIAL DIAGNOSES?
WORKUP?

20. POLYARTERITIS NODOSA Clinical manifestations
FUO, mononeuritis multiplex
depends on variety of vessels involved
multiple systems affected, including peripheral nerves, mesenteric vessels, heart and brain
often associated with p-ANCA in adults but classis PAN is ANCA negative.
Diagnosis: Biopsy of tissue in symptomatic sites is highly sensitive and specific and ANGIOGRAPHY for dilatation of arteries.
Differential diagnosis
HSP
Wegener’s
good pasture’s
Churg strauss

21. POLYARTERITIS NODOSA cont...
Labs:
ESR
abnormal urine
Hep B and C serology (cause 10-30%)
increased B-cell lymphocytes
hydralazine, allopurinol, penicillamine causes
Therapy
Steroids
cytoxan
prostacyclin

22. Doctor, Doctor, my teenage son coughed up blood
15 y/o male with hx of chronic sinusitis and congestion, here with complaints of hemoptysis and dyspnea but stable now.
QUESTIONS?
DIFFERENTIAL DIAGNOSIS?
WORKUP

24. WEGENER’S GRANULOMATOSIS
Differential diagnosis
sarcoid TB
Churg Strauss
Epidemiology of WGN
mostly kidney and lung with granuolomas
adults (4th and 5th decades) >>>kids
caucasians >>>noncaucasian
initially with respiratory symptoms and then renal insufficiency

25. WEGENER’S GRANULOMATOSIS
Diagnosis: Clinical findings of sinusitis and pulm finding, order high resolution CT. Check ANCA: (90% of WGN have the marker) with 70% sensitivity
Bx:
lung: granulomas
renal: 10% with granulomas, segmental necrotizing GN
RX: rapid therapy needed
Steroids
cyclophosphamide

26. OTHERS
Behcet’s disease =>f/u CT scan of brain for ocular involvement.
Cogan syndrome: autoimmune hearing loss, aortitis and keratitis
Buerger’s Disease in a male <40 yrs smoker with claudication, rest pain
SLE arteritis
Temporal arteritis with polymyalgia rheumatica
Churg-Strauss Syndrome

27. Behcet’s
Cogan’s

28. References: Nelson’s Pediatrics Harrison’s Internal Medicine
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